title: Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case.
abstract: Ectopic adrenocortical neoplasms arising in the nervous system are very rare. We encountered an intradural, extramedullary case of an adrenocortical neoplasm of indeterminate malignant potential affecting a spinal nerve root in the distal lumbar region of a 5-month-old girl. The lesion recurred 6 months after the initial gross total resection. The tumor in both resections had increased mitotic activity (5/10 high power fields) and MIB-1 labeling indices of 23% and 33% at initial resection and recurrence, respectively. Both tumors demonstrated gains of chromosomes 5 and 12 by interphase cytogenetics, whereas insulin growth factor 2 was identified in the recurrent tumor by immunohistochemistry. This report demonstrates that ectopic adrenocortical tumors in the nervous system may exhibit clinicopathologic and cytogenetic features suggestive of adrenocortical carcinoma.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA. rodriguez.fausto@mayo.edu
authors.forename: Fausto J
authors.initials: FJ
authors.lastname: Rodriguez
authors.forename: Bernd W
authors.initials: BW
authors.lastname: Scheithauer
authors.forename: Lori A
authors.initials: LA
authors.lastname: Erickson
authors.forename: Robert B
authors.initials: RB
authors.lastname: Jenkins
authors.forename: Caterina
authors.initials: C
authors.lastname: Giannini
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D008279:Magnetic Resonance Imaging; D046529:Microscopy, Electron, Transmission; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D013126:Spinal Nerve Roots / Q000378:metabolism / Q000473:pathology*